Clinical and magnetic resonance imaging manifestations of neurosarcoidosis

Semin Arthritis Rheum. 2005 Feb;34(4):649-61. doi: 10.1016/j.semarthrit.2004.07.011.


Objectives: To describe clinical and neuroimaging manifestations of neurosarcoidosis in a cohort of 21 patients.

Patients and methods: We reviewed records of 21 patients with sarcoidosis and central nervous system (CNS) manifestations referred to Cooper University Hospital, with emphasis on neuroimaging findings and associated clinical and laboratory evidence of sarcoidosis. Nineteen patients were categorized as having "definite," "probable," or "possible" neurosarcoidosis, while 1 had associated CNS vasculitis and another had Hodgkins lymphoma with cauda equina syndrome.

Results: The most common manifestations included myelopathy, cranial neuropathies, and encephalopathy. In 6 patients, CNS biopsy showed sterile, noncaseating granuloma (NCG), while in the remainder, the diagnosis was established through a combination of clinical, radiographic, and laboratory findings. Notably, 10 patients developed acute neurological emergencies, including seizures, spinal cord compression, and increased intracranial pressure. Findings on magnetic resonance imaging (MRI) included a variety of manifestations, including isolated mass lesion, diffuse intraparenchymal inflammatory lesions in the brain and spinal cord, leptomeningeal enhancement, hydrocephalus, and intracranial hemorrhage.

Conclusions: Sarcoidosis is associated with diverse neurological manifestations and neuroimaging findings. The diagnosis of isolated CNS sarcoidosis requires a biopsy to document the presence of sterile NCG and to exclude neoplasms and other granulomatous diseases. When a biopsy of the CNS is not possible, a diagnosis of neurosarcoidosis can reasonably be supported in many patients by MRI findings and exclusion of other disorders.

Relevance: Optimum management of patients with neurosarcoidosis relies on the ability of clinicians to recognize the broad spectrum of clinical and neuroimaging manifestations of the disorder.

MeSH terms

  • Adult
  • Aged
  • Brain / pathology
  • Brain Diseases / diagnosis
  • Cranial Nerve Diseases / diagnosis
  • Female
  • Humans
  • Magnetic Resonance Imaging*
  • Male
  • Middle Aged
  • Nervous System Diseases / complications*
  • Nervous System Diseases / diagnosis*
  • Retrospective Studies
  • Sarcoidosis / complications*
  • Sarcoidosis / diagnosis*
  • Spinal Cord / pathology
  • Spinal Cord Diseases / diagnosis